China Multi-Center Cohort Study on Risk Evaluation of Arrhythmogenic Cardiomyopathy

作者：Yuekai Hu, Zhongli Chen, Zhongli Chen, Anteng Shi, Zemeng Li, Zixian Chen, Zixian Chen, Ying‐Ying Zheng, Xi Zhao, Shimo Dai, Yubi Lin, Yifei Li, Bing Yang, Xianliang Zhou, Guoliang Li, Xianliang Zhou, Shengshou Hu, Lingmin Wu, Liang Chen · 发表于：JACC Asia · 年份：2025 · DOI：10.1016/j.jacasi.2025.04.005 · 被引用次数：6 · 研究领域：Cardiovascular Effects of Exercise、Cardiac electrophysiology and arrhythmias、Eosinophilic Disorders and Syndromes

BACKGROUND: Arrhythmogenic cardiomyopathy (ACM) patients in China exhibit unique genetic and clinical characteristics. There is a lack of prognostic models specific to Chinese ACM patients. OBJECTIVES: This study aims to establish a large, national ACM patient cohort with uniformly collected, high-quality data for future risk prediction. METHODS: This study includes patients with definite or borderline ACM diagnoses, along with their genotype-positive relatives. At baseline, comprehensive data collection includes medical history, electrocardiograms, imaging data, genetic testing, and laboratory evaluations. Outcome data include heart failure events and malignant ventricular arrhythmias. RESULTS: . Imaging evaluation revealed RV dilatation in 44.6% and left ventricular dilatation in 29.8%, with a mean left ventricular ejection fraction of 53.0% ± 14.5%. Regarding outcomes, malignant ventricular arrhythmias occurred in 255 (40.1%) individuals, while 21.9% developed end-stage heart failure, including 35 individuals who died of heart failure and 101 patients who underwent heart transplantation. CONCLUSIONS: The ChinaCORE ACM (China Multi-Center Cohort Study on Risk Evaluation of Arrhythmogenic Cardiomyopathy) registry is a national, longitudinal, observational cohort study. This study contributes to expanding the understanding of the disease spectrum of Chinese ACM patients and improving prognostic predictions.